Pulmonary Tumor Thrombotic Microangiopathy: A Clinical Analysis of 30 Autopsy Cases

Uruga, Hironori; Fujii, Takeshi; Kurosaki, Atsuko; Hanada, Shigeo; Takaya, Hisashi; Miyamoto, Atsushi; Morokawa, Nasa; Homma, Sakae; Kishi, Kazuma

doi:10.2169/internalmedicine.52.9472

Cited by 130 publications

(224 citation statements)

References 19 publications

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“…Autopsy series of carcinomas suggest that the reported prevalence is 1-3% [5,8]. Most reported cases of PTTM have been described in Japan, which is likely to reflect the high prevalence of gastric carcinoma.…”

Section: Incidence/epidemiologymentioning

confidence: 96%

“…myofibroblastic) in origin [5]. Cancer cell attachment is postulated to cause intimal (endothelial cell) damage, activation of coagulation and initiation of proliferation through growth factors and cytokine release/ expression, including tissue factor, vascular endothelial growth factor (VEGF) [4,8,9] and plateletderived growth factor (PDGF) [9, Fig. 1b).…”

Section: Pathogenesismentioning

confidence: 99%

“…Usually DIC is rapid and associated with bleeding, whereas in malignancy, 'compensated DIC' can occur, wherein the rate of consumption of coagulation factors and platelets is slow; thrombotic manifestations exceed bleeding events [24]. Evidence for raised D dimer or FDPs has been a uniform finding in PTTM [8,24], with features of microangiopathic haemolytic anaemia (MAHA) or DIC in half of cases at presentation [8,[24][25][26].…”

Section: Haematological Aspectsmentioning

confidence: 99%

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Pulmonary tumour thrombotic microangiopathy

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Wells

Wort

2016

Current Opinion in Pulmonary Medicine

120

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Purpose of review Pulmonary tumour thrombotic microangiopathy (PTTM) describes tumour cell microemboli with occlusive fibrointimal remodelling in small pulmonary arteries, veins and lymphatics. Progressive vessel occlusion ultimately results in pulmonary hypertension, which is often severe and rapid in onset. PTTM is associated with carcinomas, notably gastric carcinoma, with vascular endothelial growth factor and platelet-derived growth factor signalling implicated in driving the intimal remodelling. PTTM is a rare cause of pulmonary hypertension, but given that up to a quarter of autopsy specimens from patients dying of carcinoma show evidence for PTTM, it is probably underdiagnosed AQ5 . Recent findingsUntil recently, prognosis in PTTM was universally abysmal from weeks to a few months. Diagnostic utilities include aspiration of tumour cells at wedged right heart catheterization, HRCT AQ6 findings and computed tomography-positron emission tomography (CT-PET), although definitive diagnosis requires histological analysis. Reports of PTTM treated with a combination of targeted pulmonary vasodilator therapies, anticoagulation, specific chemotherapy and platelet-derived growth factor inhibition, for example using imatinib, suggest that these approaches can prolong survival.Summary PTTM is increasingly recognized as an important cause of pulmonary hypertension, often in patients presenting with new-onset pulmonary hypertension and as yet undiagnosed malignancy. Prospects of survival are improving with targeted combination therapy, and early recognition and diagnosis are likely to be the key factors to improve outcome.

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Section: Incidence/epidemiologymentioning

confidence: 96%

Section: Pathogenesismentioning

confidence: 99%

Section: Haematological Aspectsmentioning

confidence: 99%

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Pulmonary tumour thrombotic microangiopathy

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Wells

Wort

2016

Current Opinion in Pulmonary Medicine

120

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“…Conventional pulmonary angiography may show occlusion of distal vessels but without abrupt narrowing or intravascular webs suggestive of chronic thromboembolic pulmonary hypertension [5]. High-resolution computed tomography findings are non-specific but may demonstrate ground glass opacification, mosaicism (reflecting small vessel occlusion) [5], small pulmonary nodules (due to fibrointimal thickening), diffuse shadows [6], consolidation, tree-in-bud [7] and interlobular septal thickening (when pulmonary vein or lymphatics are involved). Interestingly, the recent European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines suggest that computed tomography is useful in many respects, but does not emphasis the potential role for computed tomography (and/or ultrasound) of the abdomen and pelvis to assess for malignancy [8].…”

Section: From the Authorsmentioning

confidence: 99%

“…Pulmonary vein involvement in PTTM may therefore represent a subgroup within the classification of pulmonary veno-occlusive disease [10]. The use of 18-fluorodeoxyglucose-positron emission tomography (CT-PET) has been reported to be helpful in of PTTM [7,11] but the resolution of small vessels involved may be insufficient in some cases. Importantly, one would not want to perform a lung biopsy, given the high risk of pulmonary haemorrhage in patients with pulmonary hypertension and the likely poor tolerance to anaesthesia and sedation.…”

Section: From the Authorsmentioning

confidence: 99%

Earlier diagnosis and international registries may improve outcomes in pulmonary tumour thrombotic microangiopathy

Price

Wort

2016

Eur Respir J

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Progressive Dyspnea in a Woman With Genital Skin Lesions

Teruya

Mukaigawara²,

Hirata

2020

JAMA Oncol

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An 82-year-old woman presented with dyspnea on exertion and weight gain of 5 kg over the past month. Her medical history included atrial fibrillation and lung adenocarcinoma (a lung lobectomy was performed 8 years ago). Her blood pressure was 112/60 mm Hg, her heart rate was 100 beats per minute, her respiratory rate was 37 breaths per minute, her body temperature was 36.6°C, and her oxygen saturation was 100% while breathing 10 L of oxygen. A cardiac examination was clinically significant for an irregular rhythm, an increased pulmonic closure sound, and jugular venous distension. Bibasilar coarse crackles and pretibial edema were also noted. A complete blood count had results within normal limits. The serum lactate dehydrogenase level was 278 U/L (to convert to microkatal per liter, multiply by 0.0167). A chest radiographic image showed bibasilar infiltrates. An electrocardiogram was notable for atrial fibrillation, and echocardiography revealed normal left ventricular function, mild right atrial and ventricular dilatation, and severe tricuspid regurgitation (transtricuspid pressure gradient, 40 mm Hg). A contrast-enhanced computed tomography image revealed bilateral ground-glass opacities and right basilar consolidations, with no evidence of thromboembolisms (Figure , A). Para-aortic and inguinal lymphadenopathies were also noted. Presumptive diagnoses of pulmonary hypertension (PH) and right-sided heart failure were made. The patient began receiving dobutamine and furosemide, but she continuously required 2 to 10 L of oxygen, and further invasive evaluations could not be performed. An echocardiogram identified aggravating PH (transtricuspid pressure gradient, 57 mm Hg). A thorough physical examination identified scaly, crusty genital skin lesions pathologically compatible with extramammary Paget disease. The patient deteriorated and died despite treatment. An autopsy was performed (Figure , B).

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Pulmonary Tumor Thrombotic Microangiopathy: A Clinical Analysis of 30 Autopsy Cases

Cited by 130 publications

References 19 publications

Pulmonary tumour thrombotic microangiopathy

Pulmonary tumour thrombotic microangiopathy

Earlier diagnosis and international registries may improve outcomes in pulmonary tumour thrombotic microangiopathy

Progressive Dyspnea in a Woman With Genital Skin Lesions

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